What’s New in Bullous Disease?

John Zone, MD

Dr Zone, a leading expert in immunodermatology, presented some of the latest data on the management of bullous diseases.

Therapies discussed included:

• Rituxumab (off label) -Pemphigus/mucus membrane pemphigoid
• IVIg (FDA approved)-Pemphigus/Pemphigoid
• IVIg (FDA approved)-Controversial for Toxic epidermal necrolysis (TEN)/Stevens-Johnson syndrome (SJS)
• IVIg (off label)-Scleromyxedema
• IgE and Omalizumab (off label)

Rituximab has demonstrated positive efficacy for the treatment of both pemphigus and mucus membrane pemphigoid.  Intravenous Immune Globulin (IVIg) is FDA now approved for a variety of dermatologic disorders, and because of the thousands of donors, it is rather accessible.   The efficacy of high dose IVIg in the treatment of TEN has been studied and it has demonstrated positive results in adults; however, it is important to keep in mind that IVIg is not benign and adverse effects can occur.

What about scleromyxedema? Scleromyxedema is a rare disease with mucin deposition in the skin and other organs with monoclonal IgG protein. In a case series report, the data on IVIg therapy for scleromyxedema was positive. Finally, Dr Zone feels that in the next ten years IgE will really “catch on.”  Omalizumab can treat diseases such as urticaria, pemphigoid, atopic dermatitis and many more disorders.